NDUFA10

Protein identityUniProt · HPA

UniProt accession

O95299

Gene

NDUFA10

Ensembl

ENSG00000130414

Chromosome

2

Canonical length

355 aa

Protein class

Disease related genes, Human disease related genes, Metabolic proteins, Predicted intracellular proteins

Subcellular location

Mitochondria

OverviewNCBI Gene

The protein encoded by this gene is a component of 42 kDa complex I, the first enzyme complex in the electron transport chain of mitochondria. This protein has NADH dehydrogenase activity and oxidoreductase activity. It transfers electrons from NADH to the respiratory chain. A mutation in this gene was found in an individual with Leigh syndrome. [provided by RefSeq, Apr 2016]

Canonical amino-acid sequenceUniProt

355 residues, UniProt reviewed canonical sequence.

>O95299|NDUFA10
     1  MALRLLKLAA TSASARVVAA GAQRVRGIHS SVQCKLRYGM WHFLLGDKAS KRLTERSRVI
    61  TVDGNICTGK GKLAKEIAEK LGFKHFPEAG IHYPDSTTGD GKPLATDYNG NCSLEKFYDD
   121  PRSNDGNSYR LQSWLYSSRL LQYSDALEHL LTTGQGVVLE RSIFSDFVFL EAMYNQGFIR
   181  KQCVDHYNEV KSVTICDYLP PHLVIYIDVP VPEVQRRIQK KGDPHEMKIT SAYLQDIENA
   241  YKKTFLPEMS EKCEVLQYSA REAQDSKKVV EDIEYLKFDK GPWLKQDNRT LYHLRLLVQD
   301  KFEVLNYTSI PIFLPEVTIG AHQTDRVLHQ FRELPGRKYS PGYNTEVGDK WIWLK

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against NDUFA10 can act on the native protein depends on physical access: surface and secreted proteins are reachable by circulating antibodies, intracellular proteins usually are not.

Antibody reachability

Intracellular

Secreted

No

Transmembrane segments

0

Mean surface accessibility (rSASA)

0.31

Highest tissue expression

310 nTPM

Expression across tissuesHPA

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about NDUFA10.

Disease | AllUniProt

Conditions NDUFA10 is implicated in, by any mechanism.

• Mitochondrial complex I deficiency, nuclear type 22 (MC1DN22) MIM:618243

Disease | GeneticClinVar

8 pathogenic / likely-pathogenic of 391 ClinVar records.

Conditions with pathogenic or likely-pathogenic variants.

• Mitochondrial complex I deficiency, nuclear type 22

Genetic constraint and essentialitygnomAD · DepMap

Does the body need this protein intact? Low LOEUF or a strong DepMap dependency means loss or blockade of the protein is likely to be felt.

gnomAD LOEUF (loss-of-function intolerance)

1.1

gnomAD pLI

0

gnomAD missense Z

-0.36

DepMap mean gene effect

-0.31

DepMap dependency class

selective

Cancer expressionTCGA

Across TCGA tumor cohorts, this protein is over-expressed in roughly 4% of surveyed tumor types (aggregate summary; per-cohort expression, alteration, and survival load in the interactive view).

OntologyGO

Biological processes

• aerobic respiration
• mitochondrial electron transport, NADH to ubiquinone
• proton motive force-driven mitochondrial ATP synthesis

Molecular functions

• NADH dehydrogenase (ubiquinone) activity

Cellular components

• cytoplasm
• mitochondrial inner membrane
• mitochondrial matrix
• mitochondrion
• respiratory chain complex I

Protein domainsUniProt · Pfam · InterPro

• P-loop containing nucleoside triphosphate hydrolase
• Deoxynucleoside kinase domain
• Deoxyribonucleoside Kinase
• Deoxynucleoside kinase
• NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial

KeywordsUniProt

• Electron transport
• FAD
• Flavoprotein
• Mitochondrion
• Phosphoprotein
• Primary mitochondrial disease
• Respiratory chain
• Transit peptide
• Transport

InteractionsUniProt · HPA

Protein binding partners of NDUFA10 in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.

• NDUFA9
• NDUFB3
• NDUFS7